RT Journal Article
SR Electronic
T1 Pulmonary hypertension in antisynthetase syndrome: prevalence, etiology and survival
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP erj01563-2012
DO 10.1183/09031936.00156312
A1 Baptiste Hervier
A1 Alain Meyer
A1 Céline Dieval
A1 Yurdagul Uzunhan
A1 Hervé Devilliers
A1 David Launay
A1 Matthieu Canuet
A1 Laurent Têtu
A1 Christian Agard
A1 Jean Sibilia
A1 Mohamed Hamidou
A1 Zahir Amoura
A1 Hilario Nunes
A1 Olivier Benveniste
A1 Philippe Grenier
A1 David Montani
A1 Eric Hachulla
YR 2013
UL http://erj.ersjournals.com/content/early/2013/01/23/09031936.00156312.abstract
AB Antisynthetase syndrome (ASS) is characterized by the association of interstitial lung disease (ILD) and myositis with different anti-tRNA-synthetase antibodies. The occurrence, etiology and prognosis of pulmonary hypertension (PH) have not yet been evaluated.Among 203 consecutive patients, echocardiography (TTE) and right heart catheterization (RHC) results were retrospectively analysed in light of clinico-biological, morphological and functional parameters. Definitions of PH were based on the ESC/ERS 2009 guidelines, severe PH being defined by a mean pulmonary arterial pressure (mPAP) >35 mmHg.PH was suspected by TTE in 47 (23.2%) cases, corresponding to PH “possible” (n=27, 13.3%) or “likely” (n=20, 9.9%). RHC was performed in 21 patients, excluding PH in 5 and confirming pre-capillary PH in 16 (7.9%). Although related to ILD in all cases, pre-capillary PH was severe in 13 (81,3%) patients (mean mPAP: 46±9 mmHg), frequently associated with low cardiac index (mean 2.3±0.8 l·min−1·m−2) and high FVC/DL,CO ratio (2.5±0.6). PH was clearly associated with a lower survival (p<0.001), with a 3-year survival rate of 58%.The occurrence of PH in ASS is significant and dramatically worsens the prognosis. Although systematically associated with ILD, PH was usually severe, suggesting a specific pulmonary vascular involvement.